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Targeted therapy in recurrent clival chordoma: a case report of response to ivosidenib

Elnaz Rahbarlayegh1, Santosh Kesari1,2

  • 1The Lundquist Institute, Torrance, CA 90502, United States.

The Oncologist
|June 25, 2026
PubMed

Insights

Targeted therapy with an isocitrate dehydrogenase 1 (IDH1) inhibitor showed significant clinical benefits for a patient with recurrent clival chordoma. Molecular profiling identified the IDH1 mutation, guiding this successful individualized treatment approach.

Area of Science:

  • Oncology
  • Genomics
  • Precision Medicine

Background:

  • Genetic and molecular alterations in cancer are key therapeutic targets for personalized treatment strategies.
  • Rare tumors often have limited systemic treatment options, making molecular profiling crucial for identifying novel therapeutic opportunities.

Purpose of the Study:

  • To report a case of recurrent clival chordoma treated with targeted therapy based on molecular profiling.
  • To illustrate the potential of isocitrate dehydrogenase 1 (IDH1)-targeted therapy in patients with chordoma harboring IDH1 mutations.

Main Methods:

  • Comprehensive tumor molecular profiling was performed on a patient with multiply recurrent clival chordoma.
  • Off-label treatment with the IDH1 inhibitor ivosidenib was initiated based on the identification of an activating IDH1 p. R132C mutation.

Main Results:

  • The patient experienced a durable radiographic response with tumor reduction and a partial metabolic response on FDG-PET imaging.
  • Clinically significant improvements in neurological symptoms and quality of life were observed.
  • Treatment with ivosidenib was well tolerated.

Conclusions:

  • Molecular tumor board-guided interpretation of genomic alterations is valuable in guiding treatment decisions.
  • IDH-targeted therapy shows potential as a treatment option for select patients with recurrent chordoma harboring IDH1 mutations.
Keywords:
Clival ChordomaIDH1 mutationIvosidenibMolecular tumor boardTargeted Therapy

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